The challenge of extraabdominal desmoid tumour management in patients with Gardner's syndrome: radiofrequency ablation, a promising option

Lorenzo Cobianchi, Valentina Ravetta, Francesca T orello Viera, Claudia Filisetti, Barbara Siri, Edoardo Segalini, Marcello Maestri, Tommaso Dominioni, Mario Alessiani, Sandro Rossi, Paolo Dionigi

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3 Citations (Scopus)

Abstract

Desmoid tumours are benign, myofibroblastic stromal neoplasms common in Gardner's syndrome, which is a subtype of familial adenomatous polyposis characterized by colonic polyps, osteomas, thyroid cancer, epidermoid cysts, fibromas and sebaceous cysts. The primary treatment is surgery, followed by adjuvant radiotherapy, but the local recurrence rate is high, and wide resection can result in debilitating loss of function. We report the case of a 39-year-old man with Gardner's syndrome who had already undergone a total prophylactic colectomy. He developed desmoid tumours localized in the mesenteric root, abdominal wall and dorsal region, which were treated from 2003 through 2013 with several surgical procedures and percutaneous radiofrequency ablation. In 2008 and 2013, RFA was applied under ultrasonographic guidance to two desmoid tumours localized in the dorsal thoracic wall. The outcomes were low-grade pain and one case of superficial skin necrosis, but so far there has been no recurrence of desmoid tumours in these locations. Surgical resection remains the first-line therapy for patients with desmoid tumours, but wide resection may lead to a poor quality of life. Radiofrequency ablation is less invasive and expensive and is a possible therapeutic option for desmoid tumours in patients with Gardner's syndrome.

Original languageEnglish
Pages (from-to)361
Number of pages1
JournalWorld Journal of Surgical Oncology
Volume12
DOIs
Publication statusPublished - 2014

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Gardner Syndrome
Aggressive Fibromatosis
Neoplasms
Epidermal Cyst
Colonic Polyps
Osteoma
Recurrence
Adjuvant Radiotherapy
Adenomatous Polyposis Coli
Fibroma
Colectomy
Abdominal Wall
Thoracic Wall
Thyroid Neoplasms
Necrosis
Therapeutics
Quality of Life
Pain
Skin

ASJC Scopus subject areas

  • Medicine(all)

Cite this

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title = "The challenge of extraabdominal desmoid tumour management in patients with Gardner's syndrome: radiofrequency ablation, a promising option",
abstract = "Desmoid tumours are benign, myofibroblastic stromal neoplasms common in Gardner's syndrome, which is a subtype of familial adenomatous polyposis characterized by colonic polyps, osteomas, thyroid cancer, epidermoid cysts, fibromas and sebaceous cysts. The primary treatment is surgery, followed by adjuvant radiotherapy, but the local recurrence rate is high, and wide resection can result in debilitating loss of function. We report the case of a 39-year-old man with Gardner's syndrome who had already undergone a total prophylactic colectomy. He developed desmoid tumours localized in the mesenteric root, abdominal wall and dorsal region, which were treated from 2003 through 2013 with several surgical procedures and percutaneous radiofrequency ablation. In 2008 and 2013, RFA was applied under ultrasonographic guidance to two desmoid tumours localized in the dorsal thoracic wall. The outcomes were low-grade pain and one case of superficial skin necrosis, but so far there has been no recurrence of desmoid tumours in these locations. Surgical resection remains the first-line therapy for patients with desmoid tumours, but wide resection may lead to a poor quality of life. Radiofrequency ablation is less invasive and expensive and is a possible therapeutic option for desmoid tumours in patients with Gardner's syndrome.",
author = "Lorenzo Cobianchi and Valentina Ravetta and Viera, {Francesca T orello} and Claudia Filisetti and Barbara Siri and Edoardo Segalini and Marcello Maestri and Tommaso Dominioni and Mario Alessiani and Sandro Rossi and Paolo Dionigi",
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TY - JOUR

T1 - The challenge of extraabdominal desmoid tumour management in patients with Gardner's syndrome

T2 - radiofrequency ablation, a promising option

AU - Cobianchi, Lorenzo

AU - Ravetta, Valentina

AU - Viera, Francesca T orello

AU - Filisetti, Claudia

AU - Siri, Barbara

AU - Segalini, Edoardo

AU - Maestri, Marcello

AU - Dominioni, Tommaso

AU - Alessiani, Mario

AU - Rossi, Sandro

AU - Dionigi, Paolo

PY - 2014

Y1 - 2014

N2 - Desmoid tumours are benign, myofibroblastic stromal neoplasms common in Gardner's syndrome, which is a subtype of familial adenomatous polyposis characterized by colonic polyps, osteomas, thyroid cancer, epidermoid cysts, fibromas and sebaceous cysts. The primary treatment is surgery, followed by adjuvant radiotherapy, but the local recurrence rate is high, and wide resection can result in debilitating loss of function. We report the case of a 39-year-old man with Gardner's syndrome who had already undergone a total prophylactic colectomy. He developed desmoid tumours localized in the mesenteric root, abdominal wall and dorsal region, which were treated from 2003 through 2013 with several surgical procedures and percutaneous radiofrequency ablation. In 2008 and 2013, RFA was applied under ultrasonographic guidance to two desmoid tumours localized in the dorsal thoracic wall. The outcomes were low-grade pain and one case of superficial skin necrosis, but so far there has been no recurrence of desmoid tumours in these locations. Surgical resection remains the first-line therapy for patients with desmoid tumours, but wide resection may lead to a poor quality of life. Radiofrequency ablation is less invasive and expensive and is a possible therapeutic option for desmoid tumours in patients with Gardner's syndrome.

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